Sigmar Stricker

A gradient of ROR2 protein stability and membrane localization confers brachydactyly type B or Robinow syndrome phenotypes (2009)

Schwarzer, Wibke, Witte, Florian, Rajab, Anna, Mundlos, Stefan, Stricker, Sigmar

Mutations in ROR2 cause dominant brachydactyly type B (BDB1) or recessive Robinow syndrome (RRS), each characterized by a distinct combination of phenotypic features. We here report a novel nonsense...

Evolution of a core gene network for skeletogenesis in chordates (2008)

Hecht, Jochen, Stricker, Sigmar, Wiecha, Ulrike, Stiege, Asita, Panopoulou, Georgia, Podsiadlowski, Lars, ...

The skeleton is one of the most important features for the reconstruction of vertebrate phylogeny but few data are available to understand its molecular origin. In mammals the Runt genes are central...

Triangular tibia with fibular aplasia associated with a microdeletion on 2q11.2 encompassing LAF4 (2008)

Steichen-Gersdorf, Elisabeth, Gaßner, I ., Superti-Furga, A ., Ullmann, Reinhard, Stricker, Sigmar, Klopocki, E ., ...

Nievergelt syndrome (NS) is an autosomal dominant mesomelic dysplasia characterized by specific deformities of the radius, ulna, fibula and a rhomboid shape of the tibia. Phenotypically overlapping...

Wnt-ligand-dependent interaction of TAK1 (TGF-beta-activated kinase-1) with the receptor tyrosine kinase Ror2 modulates canonical Wnt-signalling (2008)

Winkel, Andreas, Stricker, Sigmar, Tylzanowski, Przemko, Seiffart, Virginia, Mundlos, Stefan, Gross, Gerhard, ...

Mutations in the receptor tyrosine kinase Ror2 account for Brachydactyly type B and Robinow Syndrome. We have identified two novel factors interacting with the Ror2 intracellular domain. TAK1 (TGF-β...

Acetylcholine receptor pathway mutations explain various fetal akinesia deformation sequence disorders (2008)

Stricker, Sigmar, Becker, Jutta, Rupps, Rosemarie, Pantzar, Tapio, Miertus, Jan, ...

Impaired fetal movement causes malformations, summarized as fetal akinesia deformation sequence (FADS), and is triggered by environmental and genetic factors. Acetylcholine receptor (AChR) components...

The mutation ROR2W749X, linked to human BDB, is a recessive mutation in the mouse, causing brachydactyly, mediating patterning of joints and modeling recessive Robinow syndrome (2008)

Raz, Regina, Stricker, Sigmar, Elizabetta Gazzerro, Elizabetta, Clor, Julie L., Witte, Florian, Nistala, Harakiran, ...

Mutations in ROR2 result in a spectrum of genetic disorders in humans that are classified, depending on the nature of the mutation and the clinical phenotype, as either autosomal dominant...

Detection of novel skeletogenesis target genes by comprehensive analysis of a Runx2−/− mouse model (2007)

Hecht, Jochen, Seitz, Volkhard, Urban, Maren, Wagner, F., Robinson, P. N., Stiege, A., ...

Runx2 is an essential factor for skeletogenesis and heterozygous loss causes cleidocranial dysplasia in humans and a corresponding phenotype in the mouse. Homozygous Runx2-deficient mice lack...

Multiple roles for neurofibromin in skeletal development and growth. (2007)

Kolanczyk, Mateusz, Kossler, Nadine, Kühnisch, Jirko, Lavitas, Liron, Stricker, Sigmar, Wilkening, Ulrich, ...

Neurofibromatosis type 1 (NF1) is a prevalent genetic disorder primarily characterized by the formation of neurofibromas, café-au-lait spots and freckling. Skeletal abnormalities such as short...

A comparative analysis of Meox1 and Meox2 in the developing somites and limbs of the chick embryo. (2007)

Reijntjes, Susan, Stricker, Sigmar, Mankoo, Baljinder S.

We have examined the expression pattern of the avian Meox1 homeobox gene during early development and up to late limb bud stages. Its expression pattern indicates that it is involved in somite...

Multiple Roles for Neurofibromin in Skeletal Development and Growth (2007)

Kolanczyk, Mateusz, Kossler, Nadine, Kühnisch, Jirko, Lavitas, Liron, Stricker, Sigmar, Wilkening, Ulrich, ...

Neurofibromatosis type 1 (NF1) is a prevalent genetic disorder primarily characterized by the formation of neurofibromas, café-au-lait spots, and freckling. Skeletal abnormalities such as short...

Mammalian mitochondrial nitric oxide synthase: Characterization of a novel candidate. (2006)

Zemojtel, Tomasz, Kolanczyk, Mateusz, Kossler, Nadine, Stricker, Sigmar, Lurz, Rudi, Mikula, Ivan, ...

Recently a novel family of putative nitric oxide synthases, with AtNOS1, the plant member implicated in NO production, has been described. Here we present experimental evidence that a mammalian...

Mammalian mitochondrial nitric oxide synthase: Characterization of a novel candidate. (2006)

Zemojtel, Tomasz, Kolanczyk, Mateusz, Kossler, Nadine, Stricker, Sigmar, Lurz, Rudi, Mikula, Ivan, ...

Recently a novel family of putative nitric oxide synthases, with AtNOS1, the plant member implicated in NO production, has been described. Here we present experimental evidence that a mammalian...

Mammalian mitochondrial nitric oxide synthase: Characterization of a novel candidate. (2006)

Zemojtel, Tomasz, Kolanczyk, Mateusz, Kossler, Nadine, Stricker, Sigmar, Lurz, Rudi, Mikula, Ivan, ...

Recently a novel family of putative nitric oxide synthases, with AtNOS1, the plant member implicated in NO production, has been described. Here we present experimental evidence that a mammalian...

Induction of Macrophage Chemotaxis by Aortic Extracts of the mgR Marfan Mouse Model and a GxxPG-Containing Fibrillin-1 Fragment (2006)

Guo, Gao, Booms, Patrick, Halushka, Marc, Dietz, Harry C., Ney, Andreas, Stricker, Sigmar, ...

Background— The primary cause of early death in untreated Marfan syndrome (MFS) patients is aortic dilatation and dissection. Methods and Results— We investigated whether ascending aortic samples...

Escobar Syndrome Is a Prenatal Myasthenia Caused by Disruption of the Acetylcholine Receptor Fetal γ Subunit (2006)

Hoffmann, Katrin, Müller, Juliane S., Stricker, Sigmar, Megarbane, Andre, Rajab, Anna, Lindner, Tom H., ...

Escobar syndrome is a form of arthrogryposis multiplex congenita and features joint contractures, pterygia, and respiratory distress. Similar findings occur in newborns exposed to nicotinergic...

Expression of Type XXIII Collagen mRNA and Protein (2006)

Koch, Manuel, Veit, Guido, Stricker, Sigmar, Bhatt, Pinaki, Kutsch, Stefanie, Zhou, Peihong, ...

Collagen XXIII is a member of the transmembranous subfamily of collagens containing a cytoplasmic domain, a membrane-spanning hydrophobic domain, and three extracellular triple helical collagenous...

Comparative expression pattern of Odd-skipped related genes Osr1 and Osr2 in chick embryonic development (2006)

Stricker, Sigmar, Brieske, Norbert, Haupt, Julia, Mundlos, Stefan

Odd-skipped genes encode zinc-finger transcription factors with widespread roles in embryonic development. In Drosophila, odd-skipped acts as a pair-rule gene, while its orthologous gene in...

Cloning and expression pattern of chicken Ror2 and functional characterization of truncating mutations in Brachydactyly type B and Robinow syndrome (2006)

Stricker, Sigmar, Van Wijk, Nicole Verhey, Witte, Florian, Brieske, Norbert, Seidel, Kathrin, Mundlos, Stefan

Ror2 is a receptor tyrosine kinase mutated in the human syndromes Brachydactyly type B (BDB) and recessive Robinow syndrome (RS). In this study, we used the chick as a model to investigate the role...

Activating and deactivating mutations in the receptor interaction site of GDF5 cause symphalangism or brachydactyly type A2 (2005)

Seemann, Petra, Schwappacher, Raphaela, Kjaer, Klaus W., Krakow, Deborah, Lehmann, Katarina, Dawson, Katherine, ...

Here we describe 2 mutations in growth and differentiation factor 5 (GDF5) that alter receptor-binding affinities. They cause brachydactyly type A2 (L441P) and symphalangism (R438L), conditions...

Modulation of GDF5/BRI-b signalling through interaction with the tyrosine kinase receptor Ror2 (2004)

Sammar,Marei, Stricker,Sigmar, Schwabe,Georg C., Sieber,Christina, Hartung,Anke, Hanke,Michael, ...

The brachydactylies are a group of inherited disorders of the hands characterized by shortened digits. Mutations in the tyrosine kinase receptor Ror2 cause brachydactyly type B (BDB). Mutations in...

Modulation of GDF5/BRI-b signalling through interaction with the tyrosine kinase receptor Ror2 (2004)

Sammar, Marei, Stricker, Sigmar, Schwabe, Georg C., Sieber, Christina, Hartung, Anke, Hanke, Michael, ...

The brachydactylies are a group of inherited disorders of the hands characterized by shortened digits. Mutations in the tyrosine kinase receptor Ror2 cause brachydactyly type B (BDB). Mutations in...

A Molecular Pathogenesis for Transcription Factor Associated Poly-Alanine Tract Expansions (2004)

Albrecht, Andrea N., Kornak, Uwe, Böddrich, Annett, Süring, Kathrin, Robinson, Peter N., Stiege, Asita C., ...

Poly-alanine (Ala) tract expansions in transcription factors have been shown to be associated with human birth defects such as malformations of the brain, the digits, and other structures. Expansions...

A molecular pathogenesis for transcription factor associated poly-alanine tract expansions (2004)

Albrecht, Andrea N., Kornak, Uwe, Böddrich, Annett, Süring, Kathrin, Robinson, Peter N., Stiege, Asita C., ...

Poly-alanine (Ala) tract expansions in transcription factors have been shown to be associated with human birth defects such as malformations of the brain, the digits, and other structures. Expansions...

A Molecular Pathogenesis for Transcription Factor Associated Poly-Alanine Tract Expansions (2004)

Albrecht, Andrea N., Kornak, Uwe, Böddrich, Annett, Süring, Kathrin, Robinson, Peter N., Stiege, Asita C., ...

Poly-alanine (Ala) tract expansions in transcription factors have been shown to be associated with human birth defects such as malformations of the brain, the digits, and other structures. Expansions...

Mutations in bone morphogenetic protein receptor 1B cause brachydactyly type A2 (2003)

Lehmann,Katarina, Seemann,Petra, Stricker,Sigmar, Sammar,Marai, Meyer,Birgit, Suering,Katrin, ...

Brachydactyly (BD) type A2 is an autosomal dominant hand malformation characterized by shortening and lateral deviation of the index fingers and, to a variable degree, shortening and deviation of the...

Expression of galectin-3 in skeletal tissues is controlled by Runx2 (2003)

Stock,Michael, Schafer,Henning, Stricker,Sigmar, Gross,Gerhard, Mundlos,Stefan, Otto,Florian

The -galatoside-specific lectin galectin-3 is expressed in vivo in osteoblasts as well as in epiphyseal cartilage. Here we show that in vitro, galectin-3 expression is up-regulated in the...

Expression of galectin-3 in skeletal tissues is controlled by Runx2 (2003)

Stock,Michael, Schäfer,Henning, Stricker,Sigmar, Gross,Gerhard, Mundlos,Stefan, Otto,Florian

The -galatoside-specific lectin galectin-3 is expressed in vivo in osteoblasts as well as in epiphyseal cartilage. Here we show that in vitro, galectin-3 expression is up-regulated in the...

Mutations in bone morphogenetic protein receptor 1B cause brachydactyly type A2 (2003)

Lehmann, Katarina, Seemann, Petra, Stricker, Sigmar, Sammar, Marai, Meyer, Birgit, Suering, Katrin, ...

Brachydactyly (BD) type A2 is an autosomal dominant hand malformation characterized by shortening and lateral deviation of the index fingers and, to a variable degree, shortening and deviation of the...

Expression of galectin-3 in skeletal tissues is controlled by Runx2 (2003)

Stock, Michael, Schäfer, Henning, Stricker, Sigmar, Gross, Gerhard, Mundlos, Stefan, Otto, Florian

The -galatoside-specific lectin galectin-3 is expressed in vivo in osteoblasts as well as in epiphyseal cartilage. Here we show that in vitro, galectin-3 expression is up-regulated in the...

Expression of galectin-3 in skeletal tissues is controlled by Runx2 (2003)

Stock, Michael, Schafer, Henning, Stricker, Sigmar, Gross, Gerhard, Mundlos, Stefan, Otto, Florian

The -galatoside-specific lectin galectin-3 is expressed in vivo in osteoblasts as well as in epiphyseal cartilage. Here we show that in vitro, galectin-3 expression is up-regulated in the...

Role of Runx Genes in Chondrocyte Differentiation (2002)

Stricker,Sigmar, Fundele,Reinald, Vortkamp,Andrea, Mundlos,Stefan

Runx2/Cbfa1 plays a central role in skeletal development as demonstrated by the absence of osteoblasts/bone in mice with inactivated Runx2/Cbfa1 alleles. To further investigate the role of Runx2 in...

Role of Runx Genes in Chondrocyte Differentiation (2002)

Stricker,Sigmar, Fundele,Reinald, Vortkamp,Andrea, Mundlos,Stefan

Runx2/Cbfa1 plays a central role in skeletal development as demonstrated by the absence of osteoblasts/bone in mice with inactivated Runx2/Cbfa1 alleles. To further investigate the role of Runx2 in...

Role of Runx Genes in Chondrocyte Differentiation (2002)

Stricker,Sigmar, Fundele,Reinald, Vortkamp,Andrea, Mundlos,Stefan

Runx2/Cbfa1 plays a central role in skeletal development as demonstrated by the absence of osteoblasts/bone in mice with inactivated Runx2/Cbfa1 alleles. To further investigate the role of Runx2 in...

The synpolydactyly homolog (spdh) mutation in the mouse – a defect in patterning and growth of limb cartilage eleme (2002)

Albrecht,Andrea N., Schwabe,Georg C., Stricker,Sigmar, Böddrich,Annett, Wanker,Erich E., Mundlos,Stefan

We have investigated the recessive mouse mutant synpolydactyly homolog (spdh) as a model for human synpolydactyly (SPD). As in human SPD, the spdh phenotype consists of central polydactyly,...

The synpolydactyly homolog (spdh) mutation in the mouse – a defect in patterning and growth of limb cartilage eleme (2002)

Albrecht,Andrea N., Schwabe,Georg C., Stricker,Sigmar, Böddrich,Annett, Wanker,Erich E., Mundlos,Stefan

We have investigated the recessive mouse mutant synpolydactyly homolog (spdh) as a model for human synpolydactyly (SPD). As in human SPD, the spdh phenotype consists of central polydactyly,...

Role of Runx Genes in Chondrocyte Differentiation (2002)

Stricker, Sigmar, Fundele, Reinald, Vortkamp, Andrea, Mundlos, Stefan

Runx2/Cbfa1 plays a central role in skeletal development as demonstrated by the absence of osteoblasts/bone in mice with inactivated Runx2/Cbfa1 alleles. To further investigate the role of Runx2 in...

The synpolydactyly homolog (spdh) mutation in the mouse – a defect in patterning and growth of limb cartilage eleme (2002)

Albrecht, Andrea N., Schwabe, Georg C., Stricker, Sigmar, Böddrich, Annett, Wanker, Erich E., Mundlos, Stefan

We have investigated the recessive mouse mutant synpolydactyly homolog (spdh) as a model for human synpolydactyly (SPD). As in human SPD, the spdh phenotype consists of central polydactyly,...

Role of Runx Genes in Chondrocyte Differentiation (2002)

Stricker, Sigmar, Fundele, Reinald, Vortkamp, Andrea, Mundlos, Stefan

Runx2/Cbfa1 plays a central role in skeletal development as demonstrated by the absence of osteoblasts/bone in mice with inactivated Runx2/Cbfa1 alleles. To further investigate the role of Runx2 in...

Role of Runx Genes in Chondrocyte Differentiation (2002)

Stricker, Sigmar, Fundele, Reinald, Vortkamp, Andrea, Mundlos, Stefan

Runx2/Cbfa1 plays a central role in skeletal development as demonstrated by the absence of osteoblasts/bone in mice with inactivated Runx2/Cbfa1 alleles. To further investigate the role of Runx2 in...

The synpolydactyly homolog (spdh) mutation in the mouse – a defect in patterning and growth of limb cartilage eleme (2002)

Albrecht, Andrea N., Schwabe, Georg C., Stricker, Sigmar, Böddrich, Annett, Wanker, Erich E., Mundlos, Stefan

We have investigated the recessive mouse mutant synpolydactyly homolog (spdh) as a model for human synpolydactyly (SPD). As in human SPD, the spdh phenotype consists of central polydactyly,...

Mutations in bone morphogenetic protein receptor 1B cause brachydactyly type A2

Lehmann, Katarina, Seemann, Petra, Stricker, Sigmar, Sammar, Marai, Meyer, Birgit, Süring, Katrin, ...

Brachydactyly (BD) type A2 is an autosomal dominant hand malformation characterized by shortening and lateral deviation of the index fingers and, to a variable degree, shortening and deviation of the...

An inversion involving the mouse Shh locus results in brachydactyly through dysregulation of Shh expression

Niedermaier, Michael, Schwabe, Georg C., Fees, Stephan, Helmrich, Anne, Brieske, Norbert, Seemann, Petra, ...

Short digits (Dsh) is a radiation-induced mouse mutant. Homozygous mice are characterized by multiple defects strongly resembling those resulting from Sonic hedgehog (Shh) inactivation. Heterozygous...

Activating and deactivating mutations in the receptor interaction site of GDF5 cause symphalangism or brachydactyly type A2

Seemann, Petra, Schwappacher, Raphaela, Kjaer, Klaus W., Krakow, Deborah, Lehmann, Katarina, Dawson, Katherine, ...

Here we describe 2 mutations in growth and differentiation factor 5 (GDF5) that alter receptor-binding affinities. They cause brachydactyly type A2 (L441P) and symphalangism (R438L), conditions...

Mutations in bone morphogenetic protein receptor 1B cause brachydactyly type A2

Lehmann, Katarina, Seemann, Petra, Stricker, Sigmar, Sammar, Marai, Meyer, Birgit, Süring, Katrin, ...

Brachydactyly (BD) type A2 is an autosomal dominant hand malformation characterized by shortening and lateral deviation of the index fingers and, to a variable degree, shortening and deviation of the...

An inversion involving the mouse Shh locus results in brachydactyly through dysregulation of Shh expression

Niedermaier, Michael, Schwabe, Georg C., Fees, Stephan, Helmrich, Anne, Brieske, Norbert, Seemann, Petra, ...

Short digits (Dsh) is a radiation-induced mouse mutant. Homozygous mice are characterized by multiple defects strongly resembling those resulting from Sonic hedgehog (Shh) inactivation. Heterozygous...

Activating and deactivating mutations in the receptor interaction site of GDF5 cause symphalangism or brachydactyly type A2

Seemann, Petra, Schwappacher, Raphaela, Kjaer, Klaus W., Krakow, Deborah, Lehmann, Katarina, Dawson, Katherine, ...

Here we describe 2 mutations in growth and differentiation factor 5 (GDF5) that alter receptor-binding affinities. They cause brachydactyly type A2 (L441P) and symphalangism (R438L), conditions...

Escobar Syndrome Is a Prenatal Myasthenia Caused by Disruption of the Acetylcholine Receptor Fetal γ Subunit

Hoffmann, Katrin, Müller, Juliane S., Stricker, Sigmar, Megarbane, Andre, Rajab, Anna, Lindner, Tom H., ...

Escobar syndrome is a form of arthrogryposis multiplex congenita and features joint contractures, pterygia, and respiratory distress. Similar findings occur in newborns exposed to nicotinergic...

Evolution of a Core Gene Network for Skeletogenesis in Chordates

Hecht, Jochen, Stricker, Sigmar, Wiecha, Ulrike, Stiege, Asita, Panopoulou, Georgia, Podsiadlowski, Lars, ...

The skeleton is one of the most important features for the reconstruction of vertebrate phylogeny but few data are available to understand its molecular origin. In mammals the Runt genes are central...

Acetylcholine Receptor Pathway Mutations Explain Various Fetal Akinesia Deformation Sequence Disorders

Michalk, Anne, Stricker, Sigmar, Becker, Jutta, Rupps, Rosemarie, Pantzar, Tapio, Miertus, Jan, ...

Impaired fetal movement causes malformations, summarized as fetal akinesia deformation sequence (FADS), and is triggered by environmental and genetic factors. Acetylcholine receptor (AChR) components...